Haem 12: Myeloma etc Flashcards
What is myelomas?
plasma cell dyscrasia - cancer caused by abnormal proliferation of plasma cells
What happens in myeloma?
Clonal population of plasma cells which proliferate and produce monoclonal immunoglobulin light chains:
In blood, found as paraprotein
In urine, found as Bence Jones Protein
What is the relationship between MGUS, smoldering myeloma and multiple myeloma?
MGUS and smoldering myeloma can both progress to multiple myeloma
What is the definition of multiple myeloma + the symptoms of multiple myeloma?
Clonal bone marrow plasma cells >10% in the marrow or plasmacytoma
And end organ damage-> CRAB;
Calcium: Hypercalcaemia >2.75 Renal: Creatinine clearance <40ml/min / Creatinine >177 Anaemia Hb <100g/l Bone lesions – lytic lesions (+ monoclonal protein)
What is the definition + features of smouldering myeloma? Prognosis?
Serum monoclonal protein (IgG / IgA) >30g/l (for paraprotein as well) or Bence Jones Protein
And/or Clonal bone marrow plasma cells 10%-60% in the marrow
No end-organ damage (NO CRAB)
Most will progress to myeloma untreated
What is MGUS + Prognosis?
Monoclonal Gammopathy of Unknown Significance (MGUS)
Serum monoclonal protein <30g/l
Plasma cells <10% on Bone Marrow
No end organ damage
No evidence of other disorder
1-2% per year progress to myeloma
Differences between MGUS, Smouldering Myeloma, and Multiple Myeloma (paraprotein, plasma in BM, CRAM, Leisons on MRI, free light chain ratio, progression rate)
MGUS: Paraprotein <30 Plasma cells in BM <10% No CRAB or leisons on MRI Free lgith chain ratio <100 Progression = 1-2% to MM
Smouldering Myeloma: Paraprotein >30 Plasma cells in BM 10-60% No CRAB or leisons on MRI Free light chain ratio <100 Progression = 10% to MM
MM: Paraprotein >30 OR Plasma cells in BM >10% OR CRAB or leisons on MRI PRESENT Free light chain ratio >100 Progression = 10% to MM
What can you see in blood films in myeloma?
Rouleaux formation - stacked RBCs
plasma cells in blood film is only present in v late disease
Mx of MGUS?
Anual blood test
Mx of Smouldering Myeloma?
May not always but in most cases you would treat
Mx of MM?
Treat
What does myeloma ix involve?
Investigation of paraprotein (if low level may not do all)
Urine bence jones
Whole body imaging to rule out lytic lesions
Calcium, Renal function, Full blood count
Bone marrow
General principles of myeloma mx?
Younger patients: chemotherapy then autologous stem cell transplant
Older patients: chemotherapy then maintenance therapy
What are autologous hematopoietic stem cell transplants?
Collect patient’s own stem cells
Give them high dose chemo to destroy bone marrow (and hopefully any malignancy left)
Re-infuse stem cells
more commonly used in myelomas
What are allogenic hematopoietic stem cell transplants?
Collect relative / unrelated donor cells
Give patient high dose chemotherapy to destroy remaining cancer AND patient’s immune cells to avoid rejection
Infuse stem cells
More commonly used in leukaemias